Dietary Guidelines

Nutrition plays a very important role in the management of SMA.The more severe the type of SMA, the more likely to have nutrition related health issues, which includes difficulties with feeding, chewing, swallowing and reflux. These may lead to under nutrition as there are a lot of nutrients deficiencies. The other major issue related to nutrition is weight gain as there is less physical activity and movement. Regular diet counseling with a nutritionist is essential to overcome the feeding issues, weight management and to maintain the blood parameters as well. But in this current COVID-19 pandemic scenario it is difficult to get in touch with the health professionals on a regular basis. So a Nutrition guideline is very essential to manage the diet related issues at home.

Good dietary practices

Balanced diet is important for maintaining a good health. An ideal balanced diet provides carbohydrate, protein, fat,vitamins and minerals incorrect proportion.
  • Carbohydrates are energy giving food. Body needs energy for maintaining metabolic activity and for supporting physical work and growth.Sources -All types of cereals like Rice, flaked rice, puffed rice, atta, oats, dalia, bread, cornflakes, millets, joar, bajra.
  • Protein helps in growth and development. Dietary protein provides amino acids for the synthesis of body proteins and to replace the broken one. Protein is essential for the SMA patients to build up the muscle mass.Sources-Fish, chicken, egg, pulses, milk and milk product.
  • Fat-Dietary fat provides energy and facilitates the absorption of fat soluble vitamins. Since fat provides high energy value, little amount is needed. Sources-All type of cooking oil, butter, ghee.
  • Vitamins and minerals-These are required in a very small amount. Though all kind of vitamins and minerals are essential for our survival and movements of the muscles, but emphasis should be given on Calcium and vitamin D for the formation of strong bones.
  • Sources of Vitamin D are egg, sea fish, fortified breakfast cereal. The body is also able to produce some Vitamin D in the skin using sunlight.
  • Sources of calcium are milk and milk products, small fish, leafy vegetables.
  • Vitamin C and zinc are important for immunity. Sources are all kind of fruits and vegetables.
  • Diets rich in fiber are recommended to promote gastric motility and reduce constipation. Sources are green and leafy vegetables and fruits.
  • Adequate fluids are needed to maintain hydration and gastric motility.
  • Extra sugar and salt must be avoided to prevent obesity related complications like Diabetes and Hypertension.

Weight management

The requirement of calorie is different for different individual depending on their weight and physical activity. So the target will be to restrict the food items which cause weight gain. Such as,
  • Restrict the quantity of rice/roti and cooking oil.
  • Avoid fried/junk foods, ghee/ butter, sugar, honey, sweets, chocolates, colas, candies,packaged/canned foods.
  • Give whole fruit instead of fruit juice.
  • Read food label of all packaged food to avoid added sugar, trans fat, extra salt.

Rules to minimise reflux and other gastrointestinal complications

  • Easily digestible food is the key to avoid reflux. Low fat rice based products are the best.
  • Avoid irritating and sticky foods like chocolate, peppermint, spicy & deep fried foods,citrus fruit, cheese, cola etc.
  • Fasting is not encouraged
  • Give small meals throughout the day.
  • Constipation must be avoided to prevent indigestion. Green vegetables and fruits must be there in daily diet. Make sure to supply enough fluid for proper digestion.
  • Make foods soft and small pieces to avoid chewing/swallowing difficulties.

Management to avoid aspiration

  • In case of chance of aspiration stop giving thin/clear liquids like water and juices
  • Those who cannot sit properly, prop up the bed at 20-30 degree angle while feeding to avoid choking.
  • Give semisolid easily digestible food.

Use of supplements

In case of severe feeding difficulties supplements must be given either orally or through GI tube.It has been evident that AA diet (free form of amino acid supplement which can be absorbed directly by the body to build the muscles, without the extra effort to breakdown dietary protein) is effective to improve the health status of the type 1/ type 2 patients in Western countries.

Here we can suggest ‘Junior Peptamen’(semi-elemental, peptide based formula) to the children aged 1 to 13yrs for better nourishment. In severe condition of type 1 patients ‘Alfamino (amino acid formula) can be given along with mother’s milk. Though more evidence based research work are essential to establish the effectiveness of these supplements.

 

 

 

 

 

 

 

 

 

 

 

Resource:

1.  What is Amino Acid diet for SMA By: Mary Bodzo
2.  Diet and Spinal Muscular Atrophy Current guideline  from UK
3.  Nutritional Practices at a Glance: Spinal Muscular AtrophyType 1 Nutrition Survey Findings Rebecca Hurst Davis etal.
     J Child Neurol.2014 November;29(11):1467-1472.doi: 10.1177/0883073813503988
4.  Clinical Dietetics Manual by Indian Dietetic Association
5.  General Nutrition Guideline for SMA children Spinal Muscular Atrophy Clinical Research Center, Columbia University
Douglas M Sproule, M.D.